CNS, BONES, WBC

HUMPHREY C. BITUN, MD
Try to answer the following questions
• At the end of the sample examination, review your answer and
compare with the answer key provided with the rationale for each
answer.
• Remember that a change of a single word in the stem of the question
can lead to change in answer.
• In answering the questions, try to eliminate the least possible answer
and then choose from the remaining choices.
• Do not answer in a hurry, think every possible answer because you
have all the time.
Begin
1. Which of the following is a cause of communicating form of
hydrocephalus?
A. meningioma C. tuberculous meningitis
B. Blood clot in the third ventricle D. loss of brain parenchyma

2. Patient sustained head injury from a motorcycle accident. Cranial

MRI revealed third cranial nerve compression by the medial aspect of
the temporal lobe. The type of herniation associated is:
A. Subfalcine C. cingulate
B. Tonsillar D. transtentorial
 Next
3. Patient presented with motor and sensory deficits in the lower
extremities as well as disturbances of bladder and bowel habits. Which
of the following neural tube defects will show the above findings?
A. Anencephaly C. encephalocele
B. Spina bifida D. myelomeningocele

4. The sudden disruption of neurologic function probably involves

dysregulation of reticular activating system:
A. Contussion C. concussion
B. laceration D. diffuse axonal damage
Next
5. Increased rate of subdural hematoma among older patients with
brain atrophy is due to ______ bridging veins:
A. thin-walled C. compressed
B. Stretched D. thrombosed

6. In the cerebral hemispheres, the border zone between what artery

distributions is at greatest risk of infarct?
A. Anterior and middle cerebral C. middle and posterior cerebral
B. Anterior and anterior communicating D. carotid and middle
cerebral
Next
7. Which of the following causes of cerebral embolism is the most
common culprit?
A. Fat from fractured bone C. carotid artery thromboemboli
B. Cardiac mural thrombi D. deep vein thrombosis

8. Embolic occlusion of the carotid bifurcation will lead to which of the

following gross morphologic findings?
A. Nonhemorrhagic infarct C. both
B. Hemorrhagic infarct D. neither
Next
9. The most frequent cause of clinically significant subarachnoid
hemorrhage is a ruptured:
A. A-V malformation C. saccular aneurysm
B. Charcot-Bouchard aneurysm D. cavernous malformation

10. Patient was a diagnosed case of meningitis. Autopsy revealed dense

exudates in the cerebral convexities. The etiologic agent is most
probably.
A. H. influenzae C. E. coli
B. Strep pneumoniae D. N. meningitidis
Next
11. Autopsy revealed necrotizing and hemorrhagic inflammation of the
inferior and medial regions of the temporal lobes and orbital gyri of the
frontal lobes. You will look for:
A. Intranuclear and intracytoplasmic inclusion
B. Round to oval eosinophilic cytoplasmic inclusion
C. Intranuclear inclusion
12. The two pathologic hallmarks of this lesions are plaques & tangles:
A. Alzheimer disease C. Multiple sclerosis
B. Parkinson disease D. Huntington disease
Next
13. Which of the following infiltrating gliomas is characterized by
mutations of PTEN tumor suppressor gene, deletions of chromosome
10, and amplification of the EGFR oncogene?
A. Glioblastoma C. ependymoma
B. Oligodendroglioma D. subependymoma

14. Bilateral acoustic schwannoma is commonly associated with:

A. NF1 C. Tuberous sclerosis
B. NF2 D. Von-Hippel Lindau
Next
15. This malignant brain tumor occurs predominantly in children and
exclusively in the cerebellum:
A. Atypical teratoid/rhabdoid tumor C. meningioma
B. Medulloblastoma D. neurofibroma

16. The most common cause of dwarfism is:

A. Achondroplasia C. osteogenesis imperfecta
B. Thanatophoric dysplasia D. osteopetrosis
Next
17. Post menopausal osteoporosis affects mainly the:
A. Femoral neck C. spine
B. Pelvis D. ribs

18. The histologic hallmark of this bone lesion is a mosaic pattern of

lamellar bone:
A. Osteporosis C. Paget disease
B. Osteopetrosis D. osteomalacia
Next
19. About 80% to 90% of the cases of culture-positive pyogenic
osteomyelitis is caused by:
A. E. coli C. Klebsiella
B. Pseudomonas D. S. aureus

20. Benign bone forming tumor more than 2cm diameter involving the
posterior spine:
A. Osteoid osteoma C. osteosarcoma
B. Osteoblastoma D. osteochondroma
Next
21. The formation of neoplastic bone with a fine, lace-like architecture
by the anaplastic tumor cells is diagnostic of:
A. Osteoid osteoma C. osteosarcoma
B. Osteoblastoma D. osteocrondroma

22. Common sites affected by chondrosarcoma EXCEPT:

A. Pelvis C. ribs
B. Shoulder D. femur
Next
23. Of all bone sarcomas, it have the youngest average age at
presentation.
A. Osteoclastoma C. Chondrosarcoma
B. Osteosarcoma D. Ewing sarcoma family tumors

24. CT or MRI may demonstrate internal septa and characteristic fluid-

fluid levels:
A. Aneurysmal bone cyst C. giant cell tumor
B. Fibrous dysplasia D. nonossifying fibroma
Next
25. Loose bodies (joint mice) are seen in:
A. Rheumatoid arthritis C. gouty arthritis
B. Osteoarthritis D. infectious arthritis

26. The pathognomonic hallmark of gout is:

A. Pannus C. Heberden nodes
B. Tophi D. joint exudate
Next
27. The most common disease of joints is:
A. Gout C. Rheumatoid arthritis
B. Pseudogout D. osteoarthritis

28. Reactive arthritis is compose of the following EXCEPT:

A. Arthritis C. conjunctivitis
B. Non-gonococcal urethritis or cervicitis D. enteritis
Next
29. Grape-like lesions were seen protruding out from the vagina of a
young child. It is most probably.
A. Liposarcoma C. leiomyosarcoma
B. Rhabdomyosarcoma D. synovial sarcoma

30. This tumor may show either a monophasic or biphasic type forming
gland-like structures:
A. Liposarcoma C. leiomyosarcoma
B. Rhabdomyosarcoma D. synovial sarcoma
Next
31. The most common cause of agranulocytosis is:
A. Aplastic anemia C. metastatic cancer to marrow
B. Severe sepsis D. drug toxicity

32. This form of hyperplasia may be particularly prominent in lymph

nodes draining cancers such as carcinoma of the breast.
A. Follicular C. reticular
B. Paracortical D. medullary
Next
33. The most common cancer of children is:
A. ALL C. CLL/SLL
B. AML D. CML

34. Proliferation centers are pathognomonic for:

A. ALL C. CLL/SLL
B. AML D. CML
Next
35. The most common form of NHL is:
A. Follicular C. Small lymphocytic
B. Burkitt D. Diffuse large B cell

36. Which of the following is not seen in multiple myeloma?

A. Smudge cells C. Russell bodies
B. Mott cells D. Dutcher bodies
Next
37. This rare but distinctive B-cell neoplasm shows fine threadlike
cytoplasmic extensions:
A. Anaplastic large-cell lymphoma C. Mycosis fungoides
B. Hairy cell leukemia D. large granular lymphocytic leukemia

38. The R-S cells are infected with EBV in about 70% of cases:
A. Nodular sclerosis C. Lymphocyte-rich
B. Mixed-cellularity D. lymphocyte depletion
Next
39. This is the most common form of HL:
A. Nodular sclerosis C. Lymphocyte-rich
B. Mixed-cellularity D. lymphocyte depletion

40. L & H variants are seen in:

A. Lymphocyte predominant C. Nodular sclerosis
B. Lymphocyte-rich D. lymphocyte depletion
Next
41. Distinctive needle-like azurophilic granules are seen in the
cytoplasm in many cases:
A. AML C. CML
B. ALL D. CLL

42. Philadelphia chromosome can be demonstrated in more than 90%

of cases.
A. AML C. CML
B. ALL D. CLL
Next
43. The presence of Birbeck granules in the cytoplasm is characteristic:
A. Primary myelofibrosis C. Langerhans cell histiocytosis
B. Essential thrombocytosis D. Multiple myeloma

44. Nonspecific acute splenitis can be seen in the following EXCEPT:

A. TB C. IM
B. Typhoid D. Gonorrhea
Last number
45. Myasthenia gravis is seen in 30-45% of patients with:
A. Lymphoma C. congestive splenomegaly
B. Thymoma D. splenic rupture

END!
#1
1. Which of the following is a cause of communicating form of hydrocephalus?
A. meningioma C. tuberculous meningitis
B. Blood clot in the third ventricle D. loss of brain parenchyma

Ans. C. Hydrocephalus is excess CSF caused by either increased csf production such
as in tumor of choroid plexus, obstruction to flow such as mass/tumor in the
ventricles like ependymoma and medulloblastoma and rarely intraventricular
meningioma causing non-communicating hydrocephalus, loss of brain parenchyma
among elderly leading to hydrocephalus ex vacuo due to a wider ventricle and
lastly due to decresed csf resorption such as in TB meningitis or other infections
that may caused arachnoid fibrosis impeding normal csf resorption causing
communicating form of hydrocephalus.
#2
2. Patient sustained head injury from a motorcycle accident. Cranial MRI revealed third
cranial nerve compression by the medial aspect of the temporal lobe. The type of
herniation associated is:
A. Subfalcine C. cingulate
B. Tonsillar D. transtentorial
Ans. D Herniation results from increased ICP due to SOL in the form of blood clots, tumor
mass or edema. Head injury affecting the fronto-parieta bones with accumulation of blood
or edema pushes the against the falx cerebri can compress the cingulate gyrus or
subfalcine affecting the anterior cerebral artery. On the other hand, generalized cerebral
edema or any significant mass effect pushing the cerebellum against the free border of the
foramen Magnum causes compression of the cerebellar tonsils and brain stem causing
central respiratory arrest from tonsillar herniation. When the medial portion of the
temporal lobe is compressed against the tentorium cerebelli it compresses the uncus
leading to uncal/mesial temporal/transtentorial herniation associated with compression of
the CNIII leading to aniscoria and compression of posterior cerebral artery branches
associated with Duret hemorrhages.
#3
3. Patient presented with motor and sensory deficits in the lower extremities as
well as disturbances of bladder and bowel habits. Which of the following neural
tube defects will show the above findings?
A. Anencephaly C. encephalocele
B. Spina bifida D. myelomeningocele
Ans. D Anencephaly is failure of the anterior end of the neural tube to close leading
to absence of calvarium and brain parenchyma with just remnants of area
cerebrovasculosa. Spina bifida is mild to severe with flattened disorganized
segment of spinal cord associated with an overlying meningeal outpouching.
Encephalocele is a diverticulum of malformed CNS tissue extending through a
defect in the cranium most often in the occipital region or posterior fossa.
Myelomeningocele is extension of CNS tissue through a defect in the vertebral
column most commonly in the lumbosacral region presenting with the above
clinical deficits.
#4
4. The sudden disruption of neurologic function probably involves dysregulation of reticular
activating system:
A. Contusion C. concussion
B. laceration D. diffuse axonal damage
Ans. C Contussion and laceration are due to direct parenchymal injury. Contussion is analogous to
bruishing of soft tissue while lacerations are tearing of tissue from the injury which are most
frequently in the crest of the gyri, including the frontal lobes, along orbital gyri and temporal lobes.
Diffuse axonal damage damages the integrity of the axon at the node of Ranvier, with subsequent
alteration in axoplasmic flow. Concussion is a clinical syndrome of altered consciousness secondary
to head injury typically brought about by a change in the momentum of the head (when a moving
head is suddenly arrested by impact on a rigid surface). The characteristic neurologic picture
includes instantaneous onset of transient neurologic dysfunction, including loss of consciousness,
temporary respiratory arrest, and loss of reflexes. Although neurologic recovery is complete,
amnesia for the event often persists. The pathogenesis of the sudden disruption of neurologic
function is unknown; it probably involves dysregulation of the reticular activating system in the
brainstem.
#5
5. Increased rate of subdural hematoma among older patients with brain atrophy is
due to ______ bridging veins:
A. thin-walled C. compressed
B. Stretched D. thrombosed
Ans. B Bridging veins travel from the convexities of the cerebral hemispheres
through the subarachnoid space and the subdural space to empty into the superior
sagittal sinus. Similar anatomic relationships exist with other dural sinuses. These
vessels are particularly prone to tearing along their course through the dural layers
and are the source of bleeding in most cases of subdural hematoma. In older
individuals with brain atrophy, the bridging veins are stretched, hence the
increased rate of subdural hematomas in these patients, even after relatively minor
head trauma. Infants are also particularly susceptible to subdural hematomas
because their bridging veins are thin walled. Compressed or thrombosed bridging
veins are rare.
#6
6. In the cerebral hemispheres, the border zone between what artery
distributions is at greatest risk of infarct?
A. Anterior and middle cerebral C. middle and posterior cerebral
B. Anterior and anterior communicating D. carotid and middle
cerebral
Ans. A Border zone (“watershed”) infarcts occur in the regions of the brain or
spinal cord that lie at the most distal reaches of the arterial blood supply, the
border zones between arterial territories. In the cerebral hemispheres, the
border zone between the anterior and the middle cerebral artery
distributions is at greatest risk. Damage to this region produces a sickle-
shaped band of necrosis over the cerebral convexity a few centimeters
lateral to the interhemispheric fissure. Border zone infarcts are usually seen
after hypotensive episodes.
#7
7. Which of the following causes of cerebral embolism is the most common
culprit?
A. Fat from fractured bone C. carotid artery thromboemboli
B. Cardiac mural thrombi D. deep vein thrombosis
Ans. B Embolism to the brain occurs from a variety of sources. Cardiac mural
thrombi are among the most common culprits; myocardial infarct, valvular
disease, and atrial fibrillation are important predisposing factors. Next in
importance are thromboemboli arising in arteries, most often originating
over atheromatous plaques within the carotid arteries. Other sources of
emboli include paradoxical emboli, particularly in children with cardiac
anomalies; emboli associated with cardiac surgery; and emboli of other
material (tumor, fat, or air).
#8
8. Embolic occlusion of the carotid bifurcation will lead to which of the following
gross morphologic findings?
A. Non-hemorrhagic infarct C. both
B. Hemorrhagic infarct D. neither
Ans. B The territory of distribution of the middle cerebral artery— the direct
extension of the internal carotid artery—is most frequently affected by embolic
infarction; the incidence is about equal in the two hemispheres. Emboli tend to
lodge where blood vessels branch or in areas of preexisting luminal stenosis.
“Shower embolization,” as in fat embolism, may occur after fractures; affected
individuals manifest generalized cerebral dysfunction with disturbances of higher
cortical function and consciousness, often without localizing signs. Widespread
hemorrhagic lesions involving the white matter are characteristic of embolization
of bone marrow after trauma. Thrombotic occlusion leads to pale or anemic/non-
hemorrhagic infarct.
#9
9. The most frequent cause of clinically significant subarachnoid hemorrhage is a ruptured:
A. A-V malformation C. saccular aneurysm
B. Charcot-Bouchard aneurysm D. cavernous malformation
Ans. C In some instances chronic hypertension is associated with the development of minute
aneurysms, termed Charcot-Bouchard microaneurysms, which may be the site of rupture. Charcot-
Bouchard aneurysms, not to be confused with saccular aneurysms of larger intracranial vessels in
the subarachnoid space. Saccular aneurysm is the most common type of intracranial aneurysm.
Rupture of a saccular/berry aneurysm leading to clinically significant subarachnoid hemorrhage is
most frequent in the fifth decade and is slightly more frequent in females. Vascular malformations
of the brain are classified into four principal groups: arteriovenous malformations, cavernous
malformations, capillary telangiectasias, and venous angiomas. Of these, the first two are the types
associated with risk of hemorrhage and development of neurologic symptoms. Arteriovenous
malformations are the most common type of clinically significant vascular malformation. Males are
affected twice as frequently as females. The lesion often presents between the ages of 10 and 30
years as a seizure disorder, an intracerebral hemorrhage, or a subarachnoid hemorrhage. The most
common site is the territory of the middle cerebral artery, particularly its posterior branches.
#10
10. Patient was a diagnosed case of meningitis. Autopsy revealed dense exudates in the cerebral
convexities. The etiologic agent is most probably.
A. H. influenzae C. E. coli
B. Strep pneumoniae D. N. meningitidis
Ans. B Distinctive microorganisms cause acute pyogenic meningitis in various age groups:
Escherichia coli and the group B streptococci in neonates; at the other extreme of life,
Streptococcus pneumoniae and Listeria monocytogenes are most common; and Neisseria
meningitidis in adolescents and in young adults, with clusters of cases raising public health
concerns. The introduction of immunization against Haemophilus influenzae has markedly reduced
the incidence of this infection in the developed world, particularly among infants, who used to be at
high risk.
In acute meningitis, an exudate is evident within the leptomeninges over the surface of the brain.
The meningeal vessels are engorged and stand out prominently. The anatomic distribution of the
exudate varies; in H. influenzae meningitis, for example, it is usually basal, whereas in
pneumococcal meningitis it is often densest over the cerebral convexities near the sagittal sinus.
#11
11. Autopsy revealed necrotizing and hemorrhagic inflammation of the inferior and medial regions
of the temporal lobes and orbital gyri of the frontal lobes. You will look for:
A. Intranuclear and intracytoplasmic inclusion
B. Round to oval eosinophilic cytoplasmic inclusion
C. Intranuclear inclusion
Ans. C In the immunosuppressed individual, CMV most commonly causes subacute encephalitis,
which may be associated with CMV inclusion-bearing cells. The infection tends to localize in the
paraventricular subependymal regions of the brain, where it results in a severe hemorrhagic
necrotizing ventriculoencephalitis and a choroid plexitis. Any cell in the CNS (neurons, glia,
ependyma, or endothelium) may be infected. Prominent enlarged cells with intranuclear and
intracytoplasmic inclusions. Negri bodies seen in Rabies, the pathognomonic microscopic finding,
are cytoplasmic, round to oval, eosinophilic inclusions that can be found in pyramidal neurons of
the hippocampus and Purkinje cells of the cerebellum, while HSV encephalitis starts in and most
severely involves the inferior and medial regions of the temporal lobes and the orbital gyri of the
frontal lobes. The infection is necrotizing and often hemorrhagic in the most severely affected
regions. Perivascular inflammatory infiltrates are usually present, and Cowdry type A intranuclear
viral inclusion bodies may be found in both neurons and glia.
#12
12. The two pathologic hallmarks of this lesions are plaques & tangles:
A. Alzheimer disease C. Multiple sclerosis
B. Parkinson disease D. Huntington disease
Ans. A A characteristic finding in PD is pallor of the substantia nigra and locus ceruleus, which is due
to loss of the pigmented, catecholaminergic neurons in these regions. Lewy bodies may be found in
some of the remaining neurons. These are single or multiple cytoplasmic, eosinophilic, round to
elongated inclusions that often have a dense core surrounded by a pale halo. MS is a white matter
disease that is best appreciated in sections of the brain and spinal cord. In the fresh state, the
lesions are firmer than the surrounding white matter (sclerosis) and appear as well circumscribed,
somewhat depressed, glassy, gray-tan, irregularly shaped plaques. The area of demyelination often
has sharply defined borders, a feature best appreciated with stains for myelin. Huntington disease
(HD) is an autosomal dominant disease characterized by progressive movement disorders and
dementia, caused by degeneration of striatal neurons. The brain is small and shows striking atrophy
of the caudate nucleus and, less markedly at early stages, the putamen The major microscopic
abnormalities of AD are neuritic (senile) plaques and neurofibrillary tangles. There is progressive,
eventually severe, neuronal loss and reactive gliosis in the same regions that bear the burden of
plaques and tangles.
#13
13. Which of the following infiltrating gliomas is characterized by mutations of PTEN tumor
suppressor gene, deletions of chromosome 10, and amplification of the EGFR oncogene?
A. Glioblastoma C. ependymoma
B. Oligodendroglioma D. subependymoma
Ans. A The most common genetic alterations in oligodendrogliomas are mutations of the isocitrate
dehydrogenase genes (IDH1 and IDH2), which occur in up to 90% of oligodendrogliomas and
portend a better prognosis, as they do for astrocytic tumors. Deletions of portions of chromosomes
1p and 19q, typically occurring together as a codeletion, are seen in up to 80% of cases. Additional
genetic alterations occur with progression to anaplastic oligodendroglioma. The more common of
these include loss of 9p, loss of 10q, and mutations in CDKN2A. Given the association of spinal
ependymomas with NF2, it is not surprising that the NF2 gene on chromosome 22 is commonly
mutated in ependymomas in the spinal cord but not at other sites. Ependymomas do not share the
genetic alterations that are found in infiltrating gliomas, such as mutations in TP53. The classic
subtype, comprising the majority of primary glioblastoma, is characterized by mutations of the
PTEN tumor suppressor gene, deletions of chromosome 10, and amplification of the EGFR
oncogene.
#14
14. Bilateral acoustic schwannoma is commonly associated with:
A. NF1 C. Tuberous sclerosis
B. NF2 D. Von-Hippel Lindau
Ans. B Two autosomal dominant disorders, NF1 and NF2, are familial tumor syndromes
characterized by tumors of the PNS and CNS. NF1 is the more common, with a frequency of 1 in
3,000, and is characterized by neurofibromas of peripheral nerve, gliomas of the optic nerve,
pigmented nodules of the iris (Lisch nodules), and cutaneous hyperpigmented macules (café au lait
spots). NF2 is most commonly characterized by bilateral schwannomas of the vestibulocochlear
nerves (cranial nerve VIII) and multiple meningiomas. Gliomas may also occur in these patients;
typically these are ependymomas of the spinal cord. Tuberous sclerosis is an autosomal dominant
syndrome. It is characterized by the development of hamartomas and benign neoplasms involving
the brain and other tissues; the most frequent clinical manifestations are seizures, autism, and
mental retardation. Hamartomas within the CNS take the form of cortical tubers and subependymal
nodules; subependymal giant cell astrocytomas. VHL disease, Individuals with this autosomal
dominant disease develop hemangioblastomas of the CNS and cysts involving the pancreas, liver,
and kidneys, and have a propensity to develop renal cell carcinoma and pheochromocytoma.
Hemangioblastomas are most common in the cerebellum and retina, but may also occur in other
locations in the CNS.
#15
15. This malignant brain tumor occurs predominantly in children and exclusively in the
cerebellum:
A. Atypical teratoid/rhabdoid tumor C. meningioma
B. Medulloblastoma D. neurofibroma
Ans. B Atypical teratoid/rhabdoid tumor. This highly malignant tumor of young children is a
WHO grade IV tumor occurring in the posterior fossa and supratentorial compartments in
nearly equal proportions. It is characterized by divergent differentiation with epithelial,
mesenchymal, neuronal, and glial components, and often includes rhabdoid cells,
resembling those of a rhabdomyosarcoma. Medulloblastoma:This malignant embryonal
tumor occurs predominantly in children and exclusively in the cerebellum (by definition).
Neuronal and glial markers may be expressed, but the tumor is often largely
undifferentiated and corresponds to WHO grade IV. Meningiomas are predominantly
benign tumors of adults, usually attached to the dura, that arise from the meningothelial
cells of the arachnoid. Meningiomas may be found along any of the external surfaces of
the brain as well as within the ventricular system.
#16
16. The most common cause of dwarfism is:
A. Achondroplasia C. osteogenesis imperfecta
B. Thanatophoric dysplasia D. osteopetrosis
Ans. A Achondroplasia is the most common skeletal dysplasia and a major cause of dwarfism. It is an autosomal
dominant disorder resulting in retarded cartilage growth. Affected individuals have shortened proximal
extremities, a trunk of relatively normal length, and an enlarged head with bulging forehead and conspicuous
depression of the root of the nose. The skeletal abnormalities are usually not associated with changes in
longevity, intelligence, or reproductive status. It is caused by gain-of-function mutations in the FGF receptor 3
(FGFR3). Thanatophoric dysplasia is the most common lethal form of dwarfism. Affected individuals have
micromelic shortening of the limbs, frontal bossing, relative macrocephaly, a small chest cavity, and a bell-
shaped abdomen. Osteogenesis imperfecta (OI), or brittle bone disease, is a phenotypically diverse disorder
caused by deficiencies in the synthesis of type I collagen. It is the most common inherited disorder of
connective tissue. OI principally affects bone, but also impacts other tissues rich in type I collagen (joints, eyes,
ears, skin, and teeth). Osteopetrosis, also known as marble bone disease and Albers-Schönberg disease, refers
to a group of rare genetic diseases that are characterized by reduced bone resorption and diffuse symmetric
skeletal sclerosis due to impaired formation or function of osteoclasts. Due to deficient osteoclast activity,
bones involved by osteopetrosis lack a medullary canal, and the ends of long bones are bulbous
(Erlenmeyer flask deformity) and misshapen. The absence of CA2 prevents osteoclasts from acidifying the
resorption pit and solubilizing hydroxyapatite.
#17
17. Post menopausal osteoporosis affects mainly the:
A. Femoral neck C. spine
B. Pelvis D. ribs
Ans. C The term osteopenia refers to decreased bone mass, and osteoporosis is defined as
osteopenia that is severe enough to significantly increase the risk of fracture. The most common
forms of osteoporosis are the senile and postmenopausal types. Postmenopausal osteoporosis is
characterized by an acceleration of bone loss. In the decade after menopause, yearly reductions in
bone mass may reach up to 2% of cortical bone and 9% of cancellous bone. Women may lose as
much as 35% of their cortical bone and 50% of their cancellous bone by 30 to 40 years after
menopause. The hallmark of osteoporosis is histologically normal bone that is decreased in
quantity. The entire skeleton is affected in postmenopausal and senile osteoporosis, but
certain bones tend to be more severely impacted. In postmenopausal osteoporosis the
increase in osteoclast activity affects mainly bones or portions of bones that have
increased surface area, such as the cancellous compartment of vertebral bodies. The
trabecular plates become perforated, thinned, and lose their interconnections, leading to
progressive microfractures and eventual vertebral collapse.
#18
18. The histologic hallmark of this bone lesion is a mosaic pattern of lamellar bone:
A. Osteoporosis C. Paget disease
B. Osteopetrosis D. osteomalacia
Ans. C Paget disease is a disorder of increased, but disordered and structurally
unsound, bone mass. This unique skeletal disease can be divided into three
sequential phases: (1) an initial osteolytic stage, (2) a mixed osteoclastic-
osteoblastic stage, which ends with a predominance of osteoblastic activity and
evolves ultimately into (3) a final burned-out quiescent osteosclerotic stage. Paget
disease shows remarkable histologic variation over time and from site to
site. The hallmark is a mosaic pattern of lamellar bone, seen in the sclerotic phase.
This jigsaw puzzle-like appearance is produced by unusually prominent
cement lines, which join haphazardly oriented units of lamellar bone.
Osteomalacia is characterized by bone that is insufficiently mineralized. In the
developing skeleton, the manifestations are characterized by a condition known as
rickets.
#19
19. About 80% to 90% of the cases of culture-positive pyogenic osteomyelitis is caused by:
A. E. coli C. Klebsiella
B. Pseudomonas D. S. aureus
Ans.D Pyogenic osteomyelitis is almost always caused by bacterial infections. Organisms
may reach the bone by (1) hematogenous spread, (2) extension from a contiguous site, and
(3) direct implantation. In otherwise healthy children, most osteomyelitis is hematogenous
in origin and develops in the long bones. Staphylococcus aureus is responsible for 80% to
90% of the cases of culture-positive pyogenic osteomyelitis. Escherichia coli, Pseudomonas,
and Klebsiella are more frequently isolated from individuals with genitourinary tract
infections or who are intravenous drug abusers. Mixed bacterial infections are seen in the
setting of direct spread or inoculation of organisms during surgery or into open fractures.
In the neonatal period, Haemophilus influenzae and group B streptococci are frequent
pathogens, and individuals with sickle cell disease are predisposed to Salmonella infection.
In almost 50% of suspected cases, no organisms can be isolated.
#20
20. Benign bone forming tumor more than 2cm diameter involving the posterior spine:
A. Osteoid osteoma C. osteosarcoma
B. Osteoblastoma D. osteochondroma
Ans. B Osteoid osteoma and osteoblastoma are benign bone producing tumors that have identical histologic
features but differ in size, sites of origin, and symptoms. Osteoid osteomas are, by definition less than 2 cm in
diameter, and usually occur in young men in their teens and 20s. These tumors can arise in any bone but have
a predilection for the appendicular skeleton. About 50% of cases involve the femur or tibia, wherein they
typically arise in the cortex and less frequently within the medullary cavity. Osteoblastoma is larger than 2 cm
and involves the posterior spine (laminae and pedicles) more frequently; the pain is unresponsive to aspirin,
and the tumor usually does not induce a marked bony reaction. Osteosarcomas typically present as painful,
progressively enlarging masses. Sometimes a sudden fracture of the bone is the first symptom. Radiographs
usually show a large destructive, mixed lytic and blastic mass with infiltrative margins. The tumor frequently
breaks through the cortex and lifts the periosteum, resulting in reactive periosteal bone formation. The
triangular shadow between the cortex and raised ends of periosteum, known radiographically as Codman
triangle, is indicative of an aggressive tumor. It is characteristic but not diagnostic of osteosarcoma. Although
osteosarcoma is the most common primary malignant tumor of the bones, cartilage tumors account for the
majority of primary bone tumors (both benign and malignant). Osteochondroma, also known as an exostosis, is
a benign cartilage-capped tumor that is attached to the underlying skeleton by a bony stalk. It is the most
common benign bone tumor; about 85% are solitary.
#21
21. The formation of neoplastic bone with a fine, lace-like architecture by the
anaplastic tumor cells is diagnostic of:
A. Osteoid osteoma C. osteosarcoma
B. Osteoblastoma D. osteochondroma
Ans. C Osteochondromas are sessile or pedunculated, and range in size
from 1 to 20 cm. The cap is composed of benign hyaline cartilage
varying in thickness and is covered peripherally by perichondrium. The
cartilage has the appearance of disorganized growth plate and undergoes
enchondral ossification, with the newly made bone forming the inner
portion of the head and stalk. The formation of bone by the tumor cells is
diagnostic. The neoplastic bone usually has a fine, lace-like architecture
but also may be deposited in broad sheets or as primitive trabeculae. In
addition to bone, tumor cells may produce cartilage or fibrous tissue, but
these are not required for diagnosis.
#22
22. Common sites affected by chondrosarcoma EXCEPT:
A. Pelvis C. ribs
B. Shoulder D. femur
Ans. D Osteosarcoma occurs in all age groups but has a bimodal age distribution; 75% occur in
persons younger than 20 years of age. The smaller second peak occurs in older adults, who
frequently suffer from conditions known to predispose to osteosarcoma—Paget disease, bone
infarcts, and prior radiation. Overall, men are more commonly affected than women (1.6 : 1). Any
bone can be involved. The tumors usually arise in the metaphyseal region of the long bones of the
extremities, and almost 50% occur about the knee (i.e., distal femur or proximal tibia).
Chondrosarcomas commonly arise in the axial skeleton, especially the pelvis, shoulder, and ribs.
Unlike benign enchondroma, the distal extremities are rarely involved. Chondrosarcomas are
malignant tumors that produce cartilage. They are subclassified histologically as conventional
(hyaline cartilage producing), clear cell, dedifferentiated, and mesenchymal variants. Conventional
tumors are further subdivided by site as central (intramedullary) and peripheral (juxtacortical).
Conventional central tumors constitute about 90% of chondrosarcomas. Chondrosarcoma is about
half as common as osteosarcoma and is the second most common malignant matrix-producing
tumor of bone. Individuals with chondrosarcoma are usually in their 40s or older.
#23
23. Of all bone sarcomas, it have the youngest average age at presentation.
A. Osteoclastoma C. Chondrosarcoma
B. Osteosarcoma D. Ewing sarcoma family tumors
Ans. D Giant cell tumor is so named because the histology is dominated by multinucleated
osteoclast-type giant cells, giving rise to the synonym osteoclastoma. It is a relatively uncommon
benign, but locally aggressive, neoplasm. It usually arises in individuals in their 20s to 40s.
Ewing sarcoma is a malignant bone tumor characterized by primitive round cells without obvious
differentiation. Recently, Ewing sarcoma and primitive neuroectodermal tumor (PNET) have been
unified into a single category: the Ewing sarcoma family tumors (ESFT) based on shared clinical,
morphologic, biochemical and molecular features (discussed later). Although PNET demonstrates
more neuroectodermal differentiation than Ewing sarcoma, the distinction is not clinically
significant. Ewing sarcoma family tumors account for approximately 6% to 10% of primary malignant
bone tumors and follow osteosarcoma as the second most common group of bone sarcomas in
children. Of all bone sarcomas, ESFT have the youngest average age at presentation, since
approximately 80% are younger than 20 years. Boys are affected slightly more frequently than girls,
and there is a striking predilection for whites; blacks and Asians are rarely afflicted. ESFT usually
arise in the diaphysis of long tubular bones, especially the femur and the flat bones of the pelvis.
#24
24. CT or MRI may demonstrate internal septa and characteristic fluid-fluid levels:
A. Aneurysmal bone cyst C. giant cell tumor
B. Fibrous dysplasia D. non-ossifying fibroma
Ans. Giant cell tumors often destroy the overlying cortex, producing a bulging soft tissue
mass delineated by a thin shell of reactive bone. These are large, red-brown masses
that frequently undergo cystic degeneration. Histologically, the tumor consists of sheets
of uniform oval mononuclear cells and numerous osteoclast-type giant cells with 100 or
more nuclei. Aneurysmal bone cyst consists of multiple blood-filled cystic spaces separated
by thin, tan-white septa. The septa are composed of plump uniform fibroblasts,
multinucleated osteoclast-like giant cells, and reactive woven bone. The bone is lined by
osteoblasts, and its deposition typically follows the contours of the fibrous septa. The
lesions of fibrous dysplasia are well circumscribed, intramedullary, and vary greatly in size.
Larger lesions expand and distort the bone. The lesional tissue is tan-white and gritty and
is composed of curvilinear trabeculae of woven bone surrounded by a moderately
cellular fibroblastic proliferation. The curvilinear shapes of the trabeculae mimic Chinese
characters, and the bone lacks prominent osteoblastic rimming. Both fibrous cortical defect
and nonossifying fibroma produce sharply demarcated radiolucencies with a long axis of a
bone parallel to the cortex, surrounded by a thin rim of sclerosis.
#25
25. Loose bodies (joint mice) are seen in:
A. Rheumatoid arthritis C. gouty arthritis
B. Osteoarthritis D. infectious arthritis
Ans. B RA typically manifests as a symmetric arthritis principally affecting the small joints of the hand
and feet. The synovium becomes grossly edematous, thickened, and hyperplastic, transforming its
smooth contour to one covered by delicate and bulbous villi. The characteristic histologic features
include (1) synovial cell hyperplasia and proliferation; (2) dense inflammatory infiltrates (frequently
forming lymphoid follicles) of CD4+ helper T cells, B cells, plasma cells, dendritic cells, and
macrophages (3) increased vascularity due to angiogenesis; (4) fibrinopurulent exudate on the synovial
and joint surfaces; (5) osteoclastic activity in underlying bone, allowing the synovium to penetrate
into the bone and cause periarticular erosions and subchondral cysts. Together, the above
changes produce a pannus: a mass of edematous synovium, inflammatory cells, granulation tissue,
and fibroblasts that grows over the articular cartilage and causes its erosion.In the early stages
of osteoarthritis, the chondrocytes proliferate, forming clusters (so-called cloning). Concurrently, the
water content of the matrix increases and the concentration of proteoglycans decreases. The
normally horizontally arranged collagen type II fibers in the superficial zone are cleaved, yielding
fissures and clefts at the articular surface. This manifests as a granular soft articular surface.
Eventually, chondrocytes die and full-thickness portions of the cartilage are sloughed. The
dislodged pieces of cartilage and subchondral bone tumble into the joint, forming loose bodies (joint
mice).
#26
26. The pathognomonic hallmark of gout is:
A. Pannus C. Heberden nodes
B. Tophi D. joint exudate
Ans. B Gout is marked by transient attacks of acute arthritis initiated by crystallization of monosodium urate
within and around joints. Gout can be divided into primary and secondary forms, both sharing the common
feature of hyperuricemia. In the primary form (90% of cases), gout is the major manifestation of the disease
and the cause is usually unknown. In secondary gout (10% of cases), uric acid is increased because of a known
underlying disease that usually dominates the clinical picture. The distinctive morphologic changes in gout
are (1) acute arthritis, (2) chronic tophaceous arthritis, (3) tophi in various sites, and (4) gouty
nephropathy. Tophi are the pathognomonic hallmark of gout. They are formed by large aggregations of
urate crystals surrounded by an intense inflammatory reaction of foreign body giant cells. Tophi may
appear in the articular cartilage, ligaments, tendons, and bursae. Less frequently they may occur
in soft tissues (earlobes, fingertips) or kidneys. Superficial tophi can ulcerate through the overlying
skin. Bacterial infections that cause acute suppurative arthritis usually enter the joints from distant sites by
hematogenous spread. In neonates there is an increased incidence of contiguous spread from underlying
epiphyseal osteomyelitis. H. influenza arthritis predominates in children younger than 2 years of age, S. aureus
is the main causative agent in older children and adults, and gonococcus is prevalent during late adolescence
and young adulthood. Individuals with sickle cell disease are prone to infection with Salmonella at any age.
Joint aspiration is diagnostic if it yields purulent fluid in which the causal agent can be identified.
#27
27. The most common disease of joints is:
A. Gout C. Rheumatoid arthritis
B. Pseudogout D. osteoarthritis
Ans. D Osteoarthritis, also called degenerative joint disease, is characterized by degeneration of cartilage that
results in structural and functional failure of synovial joints. It is the most common type of joint disease.
Rheumatoid arthritis (RA) is a chronic inflammatory disorder of autoimmune origin that may affect many
tissues and organs but principally attacks the joints, producing a nonsuppurative proliferative and inflammatory
synovitis. Hyperuricemia can result from either overproduction or reduced excretion. The vast majority of
primary gout is caused by increased uric acid biosynthesis for unknown reasons. A small minority of patients
have overproduction because of identifiable enzymatic defects. For example, partial deficiency of hypoxanthine
guanine phosphoribosyl transferase (HGPRT) interrupts the salvage pathway, so purine metabolites cannot be
savaged and are, instead, degraded into uric acid. Complete absence of HGPRT also results in hyperuricemia,
but the significant neurologic manifestations of this condition (Lesch-Nyhan syndrome) dominate the clinical
picture so it is classified as secondary gout. Secondary gout can also be caused by increased production (e.g.,
rapid cell lysis during chemotherapy for leukemia) or decreased excretion (chronic renal disease). The
inflammation in gout is triggered by precipitation of monosodium urate (MSU) crystals into the joints, which
result in the production of cytokines that recruit leukocytes. Calcium pyrophosphate crystal deposition disease
(CPPD), also known as pseudo-gout and chondrocalcinosis, usu ally occurs in individuals older than 50 years of
age and becomes more common with increasing age, rising to a prevalence of 30% to 60% in those 85 years or
older. The sexes and races are equally affected.
#28
28. Reactive arthritis is compose of the following EXCEPT:
A. Arthritis C. conjunctivitis
B. Non-gonococcal urethritis or cervicitis D. enteritis
Ans. D Reactive arthritis is defined by a triad of arthritis, nongonococcal urethritis or cervicitis, and
conjunctivitis. Most affected individuals are men in their 20s or 30s, and more than 80% are HLA-
B27 positive. This form of arthritis also affects individuals infected with the human
immunodeficiency virus (HIV). The disease is probably caused by an autoimmune reaction initiated
by prior infection of the genitourinary system (Chlamydia) or the gastrointestinal tract (Shigella,
Salmonella, Yersinia, Campylobacter). Arthritic symptoms develop within several weeks of the
inciting bout of urethritis or diarrhea. Joint stiffness and low back pain are common early
symptoms. The ankles, knees, and feet are affected most often, frequently in an asymmetric
pattern. Synovitis of a digital tendon sheath produces the sausage finger or toe, and ossification of
tendoliga mentous insertion sites leads to calcaneal spurs and bony outgrowths. Patients with
severe chronic disease have involvement of the spine that is indistinguishable from ankylosing
spondylitis. Extraarticular involvement manifests as inflammatory balanitis, conjunctivitis, cardiac
conduction abnormalities, and aortic regurgitation. The episodes of arthritis usually wax and wane
over several weeks to 6 months. Almost 50% of affected individuals have recurrent arthritis,
tendonitis and lumbosacral pain.
#29
29. Grape-like lesions were seen protruding out from the vagina of a young child. It is most probably.
A. Liposarcoma C. leiomyosarcoma
B. Rhabdomyosarcoma D. synovial sarcoma
Ans. B Liposarcoma is one of the most common sarcomas of adulthood. It occurs mainly in people in their 50s
to 60s in the deep soft tissues of the proximal extremities and in the retroperitoneum. Liposarcomas are
histologically divided into three morphologic subtypes: Well-differentiated liposarcoma contains
adipocytes with scattered atypical spindle cells. Myxoid liposarcoma contains abundant basophilic
extracellular matrix, arborizing capillaries and primitive cells at various stages of adipocyte
differentiation reminiscent of fetal fat. Pleomorphic liposarcoma consists of sheets of anaplastic cells,
bizarre nuclei and variable amounts of immature adipocytes (lipoblasts). Rhabdomyosarcoma is a
malignant mesenchymal tumor with skeletal muscle differentiation. Three subtypes are recognized: alveolar
(20%), embryonal (60%) and pleomorphic (20%). Rhabdomyosarcoma (alveolar and embryonal) is the most
common soft tissue sarcoma of childhood and adolescence, usually appearing before age 20. Pleomorphic
rhabdomyosarcoma is seen predominantly in adults. The pediatric forms often arise in the sinuses, head and
neck and genitourinary tract, locations that do not normally contain much skeletal muscle, Rhabdomyosarcoma
is a malignant mesenchymal tumor with skeletal muscle differentiation. Three subtypes are recognized:
alveolar (20%), embryonal (60%) and pleomorphic (20%). Rhabdomyosarcoma (alveolar and embryonal) is the
most common soft tissue sarcoma of childhood and adolescence, usually appearing before age 20.
Pleomorphic rhabdomyosarcoma is seen predominantly in adults. Sarcoma botryoides, is a variant of
embryonal rhabdomyosarcoma that develops in the walls of hollow, mucosal-lined structures, such
as the nasopharynx, common bile duct, bladder, and vagina appearing as grape-like clusters. Where
the tumors abut the mucosa of an organ, they form a submucosal zone of hypercellularity
called the cambium layer.
#30
30. This tumor may show either a monophasic or biphasic type forming gland-like structures:
A. Liposarcoma C. leiomyosarcoma
B. Rhabdomyosarcoma D. synovial sarcoma
Ans. D Leiomyosarcoma accounts for 10% to 20% of soft tissue sarcomas. They occur in adults and afflict
women more frequently than men. Most develop in the deep soft tissues of the extremities and
retroperitoneum. A particularly deadly form arises from the great vessels, especially the inferior vena cava.
Leiomyosarcomas have complex genotypes that stem from underlying defects that lead to profound genomic
instability. Leiomyosarcomas present as painless firm masses. Retroperitoneal tumors may be large
and bulky and cause abdominal symptoms. They consist of eosinophilic spindle cells with
blunt-ended, hyperchromatic nuclei arranged in interweaving fascicles. Synovial sarcoma was so-
named because the first described cases arose in the soft tissues near the knee joint and a morphologic
relationship to synovium was postulated. However, this name is a misnomer, as these tumors can present in
locations (chest wall, head and neck) that lack synovium and their morphologic features are inconsistent with
an origin from synoviocytes. Synovial sarcomas account for approximately 10% of all soft tissue sarcomas and
rank as the fourth most common sarcoma. Most occur in people in their 20s to 40s. Patients usually present
with a deep-seated mass that has been present for several years. Synovial sarcomas are morphologically
monophasic or biphasic. Monophasic synovial sarcoma consists of uniform spindle cells with scant
cytoplasm and dense chromatin growing in short, tightly packed fascicles. Many tumors historically
classified as fibrosarcoma likely would be classified as synovial sarcoma today. The tumors may
calcify. The biphasic type contains, in addition to the spindle cell component above, gland-like
structures composed of cuboidal to columnar epithelioid cells.
#31
31. The most common cause of agranulocytosis is:
A. Aplastic anemia C. metastatic cancer to marrow
B. Severe sepsis D. drug toxicity
Ans. D Neutropenia, a reduction in the number of neutrophils in the blood, occurs in a
wide variety of circumstances. Agranulocytosis, a clinically significant reduction in
neutrophils, has the serious consequence of making individuals susceptible to bacterial and
fungal infections. Neutropenia can be caused by (1) inadequate or ineffective
granulopoiesis, or (2) increased destruction or sequestration of neutrophils in the
periphery. The most common cause of agranulocytosis is drug toxicity. Certain drugs, such
as alkylating agents and antimetabolites used in cancer treatment, produce agranulocytosis
in a predictable, dose-related fashion. Because such drugs cause a generalized suppression
of hematopoiesis, production of red cells and platelets is also affected. Agranulocytosis can
also occur as an idiosyncratic reaction to a large variety of agents. The roster of implicated
drugs includes aminopyrine, chloramphenicol, sulfonamides, chlorpromazine, thiouracil,
and phenylbutazone.
#32
32. This form of hyperplasia may be particularly prominent in lymph nodes draining cancers such as carcinoma of the breast.
A. Follicular C. reticular
B. Paracortical D. medullary
Ans. C Chronic immunologic stimuli produce several different patterns of lymph node reaction. Follicular hyperplasia is
caused by stimuli that activate humoral immune responses. It is defined by the presence of
large oblong germinal centers (secondary follicles), which are surrounded by a collar of small
resting naive B cells (the mantle zone). Causes of follicular hyperplasia include rheumatoid arthritis,
toxoplasmosis, and early stages of infection with HIV. This form of hyperplasia is morphologically
similar to follicular lymphoma. Features favoring a reactive (nonneoplastic) hyperplasia include (1)
preservation of the lymph node architecture, including the interfollicular T-cell zones and the
sinusoids; (2) marked variation in the shape and size of the follicles; and (3) the presence of
frequent mitotic figures, phagocytic macrophages, and recognizable light and dark zones, all of
which tend to be absent from neoplastic follicles. Paracortical hyperplasia is caused by stimuli that
trigger T-cell–mediated immune responses, such as acute viral infections (e.g., infectious
mononucleosis). The T-cell regions typically contain immunoblasts, activated T cells three to four
times the size of resting lymphocytes that have round nuclei, open chromatin, several prominent
nucleoli, and moderate amounts of pale cytoplasm. Sinus histiocytosis (also called reticular hyperplasia)
refers to an increase in the number and size of the cells that line lymphatic sinusoids.
Although nonspecific, this form of hyperplasia may be particularly prominent in lymph nodes
draining cancers such as carcinoma of the breast. The lining lymphatic endothelial cells are
markedly hypertrophied and macrophages are greatly increased in numbers, resulting in the
expansion and distension of the sinuses.
#33
33. The most common cancer of children is:
A. ALL C. CLL/SLL
B. AML D. CML
Ans. A Acute lymphoblastic leukemia/lymphomas (ALLs) are neoplasms
composed of immature B (pre-B) or T (pre-T) cells, which are referred to as
lymphoblasts. About 85% are B-ALLs, which typically manifest as childhood
acute “leukemias.” ALL is the most common cancer of children. Chronic
lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) differ
only in the degree of peripheral blood lymphocytosis. Most affected patients
have sufficient lymphocytosis to fulfill the diagnostic requirement for CLL
(absolute lymphocyte count > 5000 per mm3). CLL is the most common
leukemia of adults in the Western world.
#34
34. Proliferation centers are pathognomonic for:
A. ALL C. CLL/SLL
B. AML D. CML
Ans. C In CLL lymph nodes are diffusely effaced by an infiltrate of
predominantly small lymphocytes 6 to 12 µm in diameter with
round to slightly irregular nuclei, condensed chromatin, and scant
cytoplasm. Admixed are variable numbers of larger activated
lymphocytes that often gather in loose aggregates referred to
as proliferation centers, which contain mitotically active cells. When
present, proliferation centers are pathognomonic for CLL/SLL. The blood
contains large numbers of small round lymphocytes with scant
cytoplasm. Some of these cells are usually disrupted in the
process of making smears, producing so-called smudge cells.
#35
35. The most common form of NHL is:
A. Follicular C. Small lymphocytic
B. Burkitt D. Diffuse large B cell
Ans. D Diffuse large B-cell lymphoma (DLBCL) is the most common form of NHL. The
common features are a relatively large cell size (usually four to five
times the diameter of a small lymphocyte) and a diffuse pattern of
growth. More anaplastic tumors may even contain multinucleated cells
with large inclusion-like nucleoli that resemble Reed-Sternberg cells (the
malignant cell of Hodgkin lymphoma). Within the category of Burkitt Lymphoma
fall (1) African (endemic) Burkitt lymphoma, (2) sporadic (nonendemic) Burkitt lymphoma,
and (3) a subset of aggressive lymphomas occurring in individuals infected with HIV. All
forms of Burkitt lymphoma are highly associated with translocations of the MYC gene on
chromosome 8 that lead to increased MYC protein levels. Numerous apoptotic cells,
the nuclear remnants of which are phagocytosed by interspersed benign
macrophages. These phagocytes have abundant clear cytoplasm, creating
a characteristic “starry sky” pattern.
#36
36. Which of the following is not seen in multiple myeloma?
A. Smudge cells C. Russell bodies
B. Mott cells D. Dutcher bodies
Ans. A Multiple myeloma (plasma cell myeloma), the most important plasma cell neoplasm, usually
presents as tumorous masses scattered throughout the skeletal system. Solitary myeloma
(plasmacytoma) is an infrequent variant that presents as a single mass in bone or soft tissue.
Smoldering myeloma refers to another uncommon variant defined by a lack of symptoms and a high
plasma M component. Relatively normal-appearing plasma cells, plasmablasts with
vesicular nuclear chromatin and a prominent single nucleolus, or bizarre,
multinucleated cells may predominate. Other cytologic variants stem from the
dysregulated synthesis and secretion of Ig, which often leads to intracellular
accumulation of intact or partially degraded protein. Such variants include flame
cells with fiery red cytoplasm, Mott cells with multiple grapelike cytoplasmic
droplets, and cells containing a variety of other inclusions, including fibrils,
crystalline rods, and globules. The globular inclusions are referred to as Russell
bodies (if cytoplasmic) or Dutcher bodies (if nuclear). In advanced disease,
plasma cell infiltrates may be present in the spleen,
#37
37. This rare but distinctive B-cell neoplasm shows fine threadlike cytoplasmic extensions:
A. Anaplastic large-cell lymphoma C. Mycosis fungoides
B. Hairy cell leukemia D. large granular lymphocytic leukemia
Ans. B Hairy cell leukemia derives its picturesque name from the appearance of the
leukemic cells, which have fine hair-like projections that are best recognized under the
phase-contrast microscope. On routine peripheral blood smears, hairy cells have round,
oblong, or reniform nuclei and moderate amounts of pale blue cytoplasm with
threadlike or bleblike extensions. Mycosis fungoides and Sézary syndrome are different manifestations
of a tumor of CD4+ helper T cells that home to the skin. Clinically, the cutaneous lesions of mycosis fungoides
typically progress through three somewhat distinct stages, an inflammatory premycotic phase, a plaque phase,
and a tumor phase. Sézary syndrome is a variant in which skin involvement is manifested as a generalized
exfoliative erythroderma. In contrast to mycosis fungoides, the skin lesions rarely proceed to tumefaction, and
there is an associated leukemia of “Sézary” cells with characteristic cerebriform nuclei. Large Granular
Lymphocytic Leukemia T,-cell and NK-cell variants of this rare neoplasm are recognized, both of which occur
mainly in adults. Individuals with T-cell disease usually present with mild to moderate lymphocytosis and
splenomegaly. Lymphadenopathy and hepatomegaly are usually absent. NK-cell disease often presents in an
even more subtle fashion, with little or no lymphocytosis or splenomegaly.
#38
38. The R-S cells are infected with EBV in about 70% of cases:
A. Nodular sclerosis C. Lymphocyte-rich
B. Mixed-cellularity D. lymphocyte depletion
Ans. B In HL identification of Reed-Sternberg cells and their variants is essential for the diagnosis.
Diagnostic Reed-Sternberg cells are large cells (45 µm in diameter) with multiple nuclei or a single nucleus with multiple nuclear
lobes, each with a large inclusion-like nucleolus about the size of a small lymphocyte. Nodular Sclerosis Type. This is the most
common form of HL, constituting 65% to 70% of cases. It is characterized by the presence of lacunar
variant Reed-Sternberg cells and the deposition of collagen in bands that divide involved lymph nodes into circumscribed
nodules. This subtype is uncommonly associated with EBV. Mixed-Cellularity Type. This form of HL constitutes
about 20% to 25% of cases. Involved lymph nodes are diffusely effaced by a heterogeneous cellular
infiltrate, which includes T cells, eosinophils, plasma cells, and benign macrophages admixed with Reed-
Sternberg cells. Diagnostic Reed-Sternberg cells and mononuclear variants are usually plentiful. The Reed-Sternberg cells are
infected with EBV in about 70% of cases. Lymphocyte Depletion Type. This is the least common form of HL,
amounting to less than 5% of cases. It is characterized by a paucity of lymphocytes and a relative
abundance of ReedSternberg cells or their pleomorphic variants. The immunophenotype of the Reed-
Sternberg cells is identical to that seen in other classical types of HL. Immunophenotyping is essential,
since most tumors suspected of being lymphocyte depletion HL actually prove to be large-cell NHLs. The
Reed-Sternberg cells are infected with EBV in over 90% of cases. Lymphocyte-Rich Type. This is an uncommon form of
classical HL in which reactive lymphocytes make up the vast majority of the cellular infiltrate. In most cases, involved
lymph nodes are diffusely effaced, but vague nodularity due to the presence of residual B-cell follicles
is sometimes seen. This entity is distinguished from the lymphocyte predominance type by the presence
of frequent mononuclear variants and diagnostic Reed-Sternberg cells with a “classical” immunophenotypic
profile. It is associated with EBV in about 40% of cases.
#39
39. This is the most common form of HL:
A. Nodular sclerosis C. Lymphocyte-rich
B. Mixed-cellularity D. lymphocyte depletion
Ans. A Nodular Sclerosis Type. This is the most common form of
HL, constituting 65% to 70% of cases. It is characterized
by the presence of lacunar variant Reed-Sternberg cells and
the deposition of collagen in bands that divide involved lymph nodes into
circumscribed nodules. This subtype is uncommonly associated
with EBV. Mixed-Cellularity Type. This form of HL constitutes
about 20% to 25% of cases. Lymphocyte Depletion Type. This
is the least common form of HL, amounting to less than
5% of cases.
#40
40. L & H variants are seen in:
A. Lymphocyte predominant C. Nodular sclerosis
B. Lymphocyte-rich D. lymphocyte depletion
Ans. A Classical forms of HL, Reed-Sternberg cells undergo
a peculiar form of cell death in which the cells
shrink and become pyknotic, a process described as
“mummification.” Lymphohistiocytic variants (L&H cells) with
polypoid nuclei, inconspicuous nucleoli, and moderately
abundant cytoplasm are characteristic of the lymphocyte
predominance subtype.
#41
41. Distinctive needle-like azurophilic granules are seen in the cytoplasm in many
cases:
A. AML C. CML
B. ALL D. CLL
Ans. A The diagnosis of AML is based on the presence of at least 20% myeloid blasts
in the bone marrow. Myeloblasts have delicate nuclear chromatin, two
to four nucleoli, and more voluminous cytoplasm than
lymphoblasts. The cytoplasm often contains fine, peroxidasepositive
azurophilic granules. Auer rods, distinctive needle-like azurophilic
granules, are present in many cases; they are particularly
numerous in AML with the t(15;17) (acute promyelocytic leukemia).
Chronic myelogenous leukemia (CML) is distinguished from other
myeloproliferative disorders by the presence of a chimeric BCRABL gene derived
from portions of the BCR gene on chromosome 22 and the ABL gene on
chromosome 9.
#42
42. Philadelphia chromosome can be demonstrated in more than 90% of
cases.
A. AML C. CML
B. ALL D. CLL
Ans. C In more than 90% of cases of CML, BCR-ABL is created by a reciprocal
(9;22)(q34;q11) translocation (the so-called Philadelphia chromosome [Ph]).
In the remaining cases the BCR-ABL fusion gene is formed by cytogenetically
complex or cryptic rearrangements and must be detected by other methods,
such as fluorescence in situ hybridization or polymerase chain reaction
(PCR)based tests. The cell of origin is a pluripotent hematopoietic
stem cell.
#43
43. The presence of Birbeck granules in the cytoplasm is characteristic:
A. Primary myelofibrosis C. Langerhans cell histiocytosis
B. Essential thrombocytosis D. Multiple myeloma
Ans. C Regardless of the clinical picture, the proliferating Langerhans cells have
abundant, often vacuolated cytoplasm and vesicular nuclei containing linear
grooves or folds. The presence of Birbeck granules in the cytoplasm is
characteristic. Birbeck granules are pentalaminar tubules, often with a dilated
terminal end producing a tennis racket-like appearance, which contain the protein
langerin. Essential thrombocytosis (ET) is often associated with activating point
mutations in JAK2 (50% of cases) or MPL (5% to 10% of cases), a receptor tyrosine
kinase that is normally activated by thrombopoietin. The hallmark of primary
myelofibrosis is the development of obliterative marrow fibrosis. The replacement
of the marrow by fibrous tissue reduces bone marrow hematopoiesis, leading to
cytopenias and extensive extramedullary hematopoiesis.
#44
44. Nonspecific acute splenitis can be seen in the following EXCEPT:
A. TB C. IM
B. Typhoid D. Gonorrhea
Ans. D. Enlargement of the spleen in nonspecific acute splenitis occurs
in any blood-borne infection. The nonspecific splenic reaction in these
infections like TB, typhoid and infectious mononucleosis caused both
by the microbiologic agents themselves and by cytokines that are
released as part of the immune response.
#45
45. Myasthenia gravis is seen in 30-45% of patients with:
A. Lymphoma C. congestive splenomegaly
B. Thymoma D. splenic rupture
Ans. B Chronic venous outflow obstruction causes a form of splenic enlargement
referred to as congestive splenomegaly. Cirrhosis of the liver is the main cause of
massive congestive splenomegaly. Splenic rupture is usually precipitated by blunt
trauma. Much less often, it occurs in the apparent absence of a physical blow. Such
“spontaneous ruptures” never involve truly normal spleens but rather stem from
some minor physical insult to a spleen made fragile by an underlying condition. The
most common predisposing conditions are infectious mononucleosis, malaria,
typhoid fever, and lymphoid neoplasms. The designation “thymoma” is restricted to
tumors of thymic epithelial cells. Such tumors typically also contain benign
immature T cells (thymocytes). About 40% of thymomas present with symptoms
stemming from impingement on mediastinal structures. Another 30% to 45% are
detected in the course of evaluating patients with myasthenia gravis. The